FaCD Online Syndrome Fact Sheet

Last updated: 22 Apr 2008

Name: Adrenocortical Cancer, Multiple Primary Malignancies in Patients with

Tumor features

adrenocortical cancer
glioblastoma (multiforme)
glioma of the brain
thyroid cancer


Levine reviewed the occurrence of secondary neoplasms in children with adrenocortical cancer, these were predominantly brain tumors[1] and might have been cases of Li-Fraumeni syndrome. The risk to develop adrenal cancer is increased in thyroid cancer survivors[2,3].

General aspects to consider with respect to multiple primary tumors:
- Shared genetic (immune response, metabolic/hormonal/DNA-repair pathways) or non-genetic (chemical carcinogens, radiation, viruses, life-style) risk factors
- Therapy (radiation, chemotherapy, hormonal,...) related effects
- Possible bias because of increased surveillance and autopsy findings.


[1] Levine GW. Adrenocortical carcinoma in two children with subsequent primary tumors. Am J Dis Child 1978; 132(3):238-240.
[2] Subramanian S, Goldstein DP, Parlea L, Thabane L, Ezzat S, Ibrahim-Zada I, Straus S, Brierley JD, Tsang RW, Gafni A, Rotstein L, Sawka AM. Second primary malignancy risk in thyroid cancer survivors: a systematic review and meta-analysis. Thyroid 2007; 17(12):1277-88.
[3] Ronckers CM, McCarron P, Ron E. Thyroid cancer and multiple primary tumors in the SEER cancer registries. International journal of cancer. Journal international du cancer 2005; 117(2):281-8.